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  1. Aniridia and WAGR Syndrome: A Guide for Patients and Their Families

21. WAGR Syndrome Information Diseases Database
wagr syndrome, Disease Database Information
http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

22. WAGR Syndrome
wagr syndrome is a rare genetic syndrome in which there is a predisposition toseveral conditions, including
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Who We Are About WebMD Site Map You are in Medical Library Our Content Sources Ask A Question Clinical Trials Health Guide A-Z Health Topics Symptoms Medical Tests Medications ... For a Complete Report WAGR Syndrome Important It is possible that the main title of the report WAGR Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms
  • WAGR Complex Wilms' Tumor-Aniridia-Genitourinary Anomalies-Mental Retardation Syndrome Wilms' Tumor-Aniridia-Gonadoblastoma-Mental Retardation Syndrome
Disorder Subdivisions
  • Aniridia-Wilms' Tumor Association AWTA Aniridia-Ambiguous Genitalia-Mental Retardation AGR Triad Aniridia-Wilms' Tumor-Gonadoblastoma
General Discussion WAGR Syndrome is a rare genetic syndrome in which there is a predisposition to several conditions, including certain malignancies, distinctive eye abnormalities, and/or mental retardation. "WAGR" is an acronym for the characteristic abnormalities associated with the syndrome. The acronym stands for (W)ilms' Tumor, the most common form of kidney cancer in children; (A)niridia, partial or complete absence of the colored region of the eye(s) (iris or irides); (G)onadoblastoma, cancer of the cells that form the testes in males or the ovaries in females (gonads); and Mental (R)etardation. A combination of two or more of these conditions must be present for an individual to be diagnosed with WAGR Syndrome. The clinical picture varies, depending upon the combination of associated abnormalities. The only feature that has been present in all documented cases of WAGR Syndrome, with only one known exception, is Aniridia.

23. Mendelian: WAGR Syndrome
Third case of wagr syndrome with severe obesity and constitutional deletion ofchromosome (11)(p12p14) Morbid obesity and hyperphagia in the wagr syndrome
http://obesitygene.pbrc.edu/cgi-bin/ace/tree/ogm2k?name=WAGR Syndrome&class=Mend

24. Wagr Syndrome - Definition From Biology-Online.org
Definition and other additional information on wagr syndrome from BiologyOnline.orgdictionary.
http://www.biology-online.org/dictionary/wagr_syndrome

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wagr syndrome
(Science: syndrome) Wilms' tumour with aniridia genitourinary abnormalities , and mental retardation . It is associated with hemizygous deletions of chromosome region
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25. WAGR Syndrome Information Diseases Database
wagr syndrome, Disease Database Information. wagr syndrome. may cause orfeature + (Follow link for list.) may be a risk factor for + (Follow link for
http://www.diseasesdatabase.com/ddb14025.htm
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WAGR syndrome WAGR syndrome: Definition(s) via UMLS Code translations and terms via UMLS WAGR syndrome: specific web sites Send WAGR syndrome to medical search engines (JavaScript enabled browsers only.) If your browser has no JavaScript you can still use these:
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26. WAGR Syndrome - [Support Group]
This information is provided as a resource and does not constitute an endorsementfor any group. It is the responsibility of the reader to decide whether a
http://www.bchealthguide.org/kbase/shc/shc29wag.htm
var hwPrint=1;var hwDocHWID="shc29wag";var hwDocTitle="WAGR Syndrome";var hwRank="1";var hwSectionHWID="shc29wag-Header";var hwSource="en-caQ2_05";var hwDocType="Shc";
Self Help Clearinghouse
WAGR Syndrome
This information is provided as a resource and does not constitute an endorsement for any group. It is the responsibility of the reader to decide whether a group is appropriate for his/her needs. For evidence-based information on diseases, conditions, symptoms, treatment and wellness issues, continue searching this site.
Reaching Out - The WAGR Network
International network. 3 affiliated groups. Founded 2000.
Provides information and support to persons with WAGR Syndrome or aniridia, and their families. Literature, networking, information and referral, and phone support. Newsletter. Encourages research. Email group.
WRITE:
Reaching Out
2063 Regina
Lincoln Park, MI 48146
CALL: 313-381-4302
WEBSITE: http://www.wagr.org
VERIFIED: 5/17/2004
The above information was "verified" as correct on the date at the end of each entry. Since American Self-Help Group Clearinghouse's database is extensive but staffing is limited and information for these organizations can change, it is not possible to keep every entry in American Self-Help Group Clearinghouse database completely current and accurate. Please check with the organizations listed for the most current information. For additional information on self-help groups, please visit the American Self-Help Group Clearinghouse web site at http://www.mentalhelp.net/selfhelp

27. WAGR Syndrome
wagr syndrome is a rare genetic syndrome in which there is a predisposition toseveral conditions, including certain malignancies, distinctive eye
http://www.bchealthguide.org/kbase/nord/nord1014.htm
var hwPrint=1;var hwDocHWID="nord1014";var hwDocTitle="WAGR Syndrome";var hwRank="1";var hwSectionHWID="nord1014-Header";var hwSource="en-caQ2_05";var hwDocType="Nord";
National Organization for Rare Disorders, Inc.
WAGR Syndrome
Important
It is possible that the main title of the report WAGR Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms
  • WAGR Complex Wilms' Tumor-Aniridia-Genitourinary Anomalies-Mental Retardation Syndrome Wilms' Tumor-Aniridia-Gonadoblastoma-Mental Retardation Syndrome
Disorder Subdivisions
  • Aniridia-Wilms' Tumor Association AWTA Aniridia-Ambiguous Genitalia-Mental Retardation AGR Triad Aniridia-Wilms' Tumor-Gonadoblastoma
General Discussion
WAGR Syndrome is a rare genetic syndrome in which there is a predisposition to several conditions, including certain malignancies, distinctive eye abnormalities, and/or mental retardation. "WAGR" is an acronym for the characteristic abnormalities associated with the syndrome. The acronym stands for (W)ilms' Tumor, the most common form of kidney cancer in children; (A)niridia, partial or complete absence of the colored region of the eye(s) (iris or irides); (G)onadoblastoma, cancer of the cells that form the testes in males or the ovaries in females (gonads); and Mental (R)etardation. A combination of two or more of these conditions must be present for an individual to be diagnosed with WAGR Syndrome. The clinical picture varies, depending upon the combination of associated abnormalities. The only feature that has been present in all documented cases of WAGR Syndrome, with only one known exception, is Aniridia.

28. WAGR Syndrome (syn. Of Aniridia)
ThirdAid.com the world s first dedicated online patient to patient medical helpcommunity. i Aniridia /i is a rare congenital eye condition leading to
http://www.thirdaid.com/conditions/WAGR_Syndrome.htm

29. WAGR Syndrome Topic - Unified Search Environment
WAGR Complex MSH/EN/D017624 Contiguous Gene Syndrome, WAGR MSH/EP/D017624 MSH/EN/D017624 WAGR Complices MSH/PM/D017624 MSH/PM/D017624
http://www.use.hcn.com.au/portals/shared/subject.`WAGR Syndrome`/home.html
WAGR Syndrome Topic Tree Definition:
A contiguous gene syndrome associated with hemizygous deletions of chromosome region 11p13. The condition is marked by the combination of Wilms' tumor (W), ANIRIDIA (A), genitourinary abnormalities (G), and MENTAL RETARDATION (R). Synonyms and Source Vocabularies:
WAGR Syndrome
Wilms Tumor-Aniridia-Genitourinary Anomalies-MR Syndrome
WAGR Complex
Contiguous Gene Syndrome, WAGR
WAGR Complices Eye Abnormalities

30. Wagr Syndrome, Renal Manifestation
Complete online version of The Encyclopaedia of Medical Imaging including textand images from The Encyclopaedia of Medical Imaging s eight book volumes
http://www.amershamhealth.com/medcyclopaedia/medical/Volume IV 2/WAGR SYNDROME R

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our commitment our company Search Medcyclopaedia for: Search marked text (mark text before you click) Browse entry words starting with: A B C D ... amershamhealth.com Wagr syndrome, renal manifestation, WAGR syndrome is an acronym for a rare complex of congenital abnormalities which include Wilms tumour , aniridia, genitourinary malformation and mental retardation. It is considered a contiguous gene syndrome, because it results from a karyotypically visible interstitial deletion of a segment of chromosome 11p13. Imaging evaluation of the WAGR syndrome is in the detection of Wilms' tumour or other genitourinary malformations. Ultrasound study may be the first-line approach, but usually CT is more helpful, especially when looking for other genitourinary malformations.
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31. WAGR Syndrome - General Practice Notebook
wagr syndrome is the association of Wilms tumour with. Aniridia; Genitourinaryabnormalities; mental Retardation with microcephaly
http://www.gpnotebook.co.uk/cache/1798635554.htm
WAGR syndrome WAGR syndrome is the association of Wilms' tumour with:
  • Aniridia Genitourinary abnormalities mental Retardation with microcephaly
It is linked to abnormalities of the chromosome 11 locus p13.
Click here for more information...

32. Aniridia And WAGR Syndrome - FISH Analysis
The Kleberg Cytogenetics Laboratory offers a fluorescence in situhybridization (FISH)based assay for identifying deletions associated with isolated
http://www.bcmgeneticlabs.org/tests/cyto/aniridiawagr.html
ANIRIDIA AND WAGR SYNDROME
FISH ANALYSIS Open Page in New Window Print This Page Return to Search The Kleberg Cytogenetics Laboratory offers a fluorescence in situ hybridization (FISH)-based assay for identifying deletions associated with isolated aniridia and WAGR syndrome. Clinical Features:
Aniridia is a developmental defect of the eye that may occur sporadically or may be inherited in a family. In the majority of cases, aniridia is an isolated finding, however, it may rarely occur as part of the contiguous gene deletion syndrome, WAGR (Wilms tumor, aniridia, genitourinary anomalies and mental retardation). The features of WAGR are variable and affected individuals are at an increased risk to develop Wilms tumor. Reasons for Referral:
Patients with isolated aniridia or clinical features suggestive of WAGR syndrome may be tested for deletions of 11p13 by FISH simultaneously with high-resolution chromosomal analysis (if not previously performed). Approximately 20-30% of patients with aniridia will have an abnormality of 11p detectable by high-resolution chromosome studies. Prenatal diagnosis may be performed if an affected family member has been studied in our laboratory and shown to have a deletion detectable by FISH. Please call regarding all prenatal samples.

33. Wilms Tumor (WT1) - FISH Analysis
The following syndromes may be due to germline mutations in WT1 wagr syndrome (LINKhere for wagr syndrome is associated with deletion of WT1; however,
http://www.bcmgeneticlabs.org/tests/cyto/wilms.html
WILMS TUMOR (
FISH ANALYSIS Open Page in New Window Print This Page Return to Search The Kleberg Cytogenetics Laboratory offers a fluorescence in situ hybridization (FISH)-based assay for identifying deletions associated with Wilms tumor.
Clinical Features:
Wilms tumor is a malignancy arising from the kidneys of infants or children and may be either bilateral or unilateral. Typically, Wilms tumor presents at age 2-4 years, but may occur up to 7-8 years of age. Wilms tumor may be isolated or may occur as part of a genetic syndrome. At present, only one gene ( ) has been identified as causing Wilms tumor. The following syndromes may be due to germline mutations in : WAGR syndrome (LINK here for WAGR), Denys-Drash syndrome, Frasier syndrome and Genitourinary anomalies without renal failure. WAGR syndrome is associated with deletion of ; however, the other syndromes are primarily due to other types of mutations. Most patients with isolated Wilms tumor do not have a detectable germline mutation of Reason for Referral:
Patients with isolated Wilms tumor or with Wilms tumor and associated anomalies suggestive of a syndromal cause may be tested for deletions of by FISH. Additionally, patients with aniridia and associated anomalies may be tested for deletions of

34. Hill Health Topics A-Z - WAGR Syndrome
wagr syndrome. National Organization for Rare Disorders. Important It is possiblethat the main title of the report wagr syndrome is not the name you
http://www.healthwise.net/hillhealth/Content/StdDocument.aspx?DOCHWID=nord1014&S

35. Rare Diseases Terms - Office Of Rare Diseases
at the bottom of the record as it may contain helpful links to web sites andpublications. Additional information about wagr syndrome is available from
http://ord.aspensys.com/asp/diseases/diseaseinfo.asp?ID=5528

36. WAGR Syndrome Synonyms, Eastern Carolina
wagr syndrome Synonyms University Health Systems of Eastern Carolina servestarboro, ahoskie, edento, winsor, maxhead, dear county, outebanks counties in
http://www.uhseast.com/114581.cfm

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Health Library Health Topics Healthy Living ... Nutrition
Information on diseases and health concerns, including symptoms, treatment options, and prevention.
WAGR Syndrome
Synonyms
Disorder Subdivisions

General Discussion

Resources
...
For a Complete Report
Important
It is possible that the main title of the report WAGR Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms
  • WAGR Complex
  • Wilms' Tumor-Aniridia-Genitourinary Anomalies-Mental Retardation Syndrome
  • Wilms' Tumor-Aniridia-Gonadoblastoma-Mental Retardation Syndrome
Disorder Subdivisions
  • Aniridia-Wilms' Tumor Association
  • AWTA
  • Aniridia-Ambiguous Genitalia-Mental Retardation
  • AGR Triad
  • Aniridia-Wilms' Tumor-Gonadoblastoma
General Discussion
WAGR Syndrome is a rare genetic syndrome in which there is a predisposition to several conditions, including certain malignancies, distinctive eye abnormalities, and/or mental retardation. "WAGR" is an acronym for the characteristic abnormalities associated with the syndrome. The acronym stands for (W)ilms' Tumor, the most common form of kidney cancer in children; (A)niridia, partial or complete absence of the colored region of the eye(s) (iris or irides); (G)onadoblastoma, cancer of the cells that form the testes in males or the ovaries in females (gonads); and Mental (R)etardation. A combination of two or more of these conditions must be present for an individual to be diagnosed with WAGR Syndrome. The clinical picture varies, depending upon the combination of associated abnormalities. The only feature that has been present in all documented cases of WAGR Syndrome, with only one known exception, is Aniridia.

37. WAGR Syndrome, Eastern Carolina
wagr syndrome University Health Systems of Eastern Carolina serves tarboro,ahoskie, edento, winsor, maxhead, dear county, outebanks counties in eastern
http://www.uhseast.com/117993.cfm

Health News
Health Library Health Topics Healthy Living ... Nutrition
Self-Help Resources
Information about national and local self-help organizations and support groups.
WAGR Syndrome
This information is provided as a resource and does not constitute an endorsement for any group. It is the responsibility of the reader to decide whether a group is appropriate for his/her needs. For evidence-based information on diseases, conditions, symptoms, treatment and wellness issues, continue searching this site.
Reaching Out - The WAGR Network
International network. 3 affiliated groups. Founded 2000.
Provides information and support to persons with WAGR Syndrome or aniridia, and their families. Literature, networking, information and referral, and phone support. Newsletter. Encourages research. Email group.
WRITE:
Reaching Out
2063 Regina
Lincoln Park, MI 48146
CALL: 313-381-4302
WEBSITE: http://www.wagr.org VERIFIED: 5/17/2004 The above information was "verified" as correct on the date at the end of each entry. Since American Self-Help Group Clearinghouse's database is extensive but staffing is limited and information for these organizations can change, it is not possible to keep every entry in American Self-Help Group Clearinghouse database completely current and accurate. Please check with the organizations listed for the most current information. For additional information on self-help groups, please visit the American Self-Help Group Clearinghouse web site at http://www.mentalhelp.net/selfhelp

38. Aniridia, PAX6 Gene, WAGR Syndrome And The Risk Of...
Aniridia, PAX6 gene, wagr syndrome and the risk of neoplastic proliferation.I Ivanov MD1, A Shuper MD2, M Shohad MD3, M Snir MD4, R Weitz MD5
http://www.cancerprev.org/Journal/Issues/20/5/601/1583
Cancer Detection and Prevention  Online Home Journal ... Familial Predisposition Aniridia, PAX6 gene, WAGR syndrome and the risk of... Published in Cancer Detection and Prevention 1996; 20(5).
Aniridia, PAX6 gene, WAGR syndrome and the risk of neoplastic proliferation
I Ivanov MD , A Shuper MD , M Shohad MD , M Snir MD , R Weitz MD Dept Pediatrics, Plovdiv Higher Medical University, Plovdiv, Bulgaria; Day-care Clinic; Institute Medical Genetics; Dept Ophthalmology; Dept Pediatrics Neurology; CMCI, Petach Tikwa, Israel AIM: To appreciate the risk of neoplastic proliferation in patients with aniridia based on the progress of the mapping of the WAGR region and discovering the structure of its PAX6 gene. METHODS: Literature review. RESULTS: Aniridia develops as a result of a mutation in the PAX6 gene which is a part of the WAGR region in chromosome 11p13. Two thirds of all cases of aniridia present an isolated familial autosomal dominant disorder affecting only several eye structures. It is due to intragenic mutations in the PAX6 gene and therefore the patients carry a standard risk for neoplastic proliferation. In the other third of the aniridic patients the disorder is sporadic and some of them may display features of the contiguous gene syndrome of Wilms tumor, aniridia, genitourinary anomalies and mental retardation. About one third of the patients with sporadic aniridia develop Wilms tumor and this risk raises to 68% if an 11p13 deletion is found. Gonadal dysgenesis, a precancerous state, and gonadoblastoma may be the expression of the genitourimary anomalies, thus representing other risks for these patients.

39. Arch Ophthalmol -- A Case Of Atypical WAGR Syndrome With Anterior Segment Anomal
genitourinary anomalies, and mental retardation (wagr syndrome) are We report a case of atypical wagr syndrome with anterior segment anomaly and
http://archopht.ama-assn.org/cgi/content/extract/119/12/1855
Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress
Vol. 119 No. 12, December 2001 Featured Link E-mail Alerts Clinicopathologic Reports, Case Reports, and Small Case Series Article Options Full text PDF Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager PubMed citation Articles in PubMed by Kawase E Azuma N Articles that cite this article ISI Web of Science (3) ... Contact me when this article is cited
A Case of Atypical WAGR Syndrome With Anterior Segment Anomaly and Microphthalmos Arch Ophthalmol. Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. INTRODUCTION
Wilms tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR syndrome) are caused by the deletion of chromosome 11p13, which includes the Wilms tumor gene ( ) and the aniridia gene ) loci (MEM, No. 194070). We report a case of atypical

40. Clinical Dysmorphology - UserLogin
A 33year-old man with wagr syndrome is described with morbid obesity associatedwith This is the third report linking wagr syndrome with obesity,
http://www.clindysmorphol.com/pt/re/mcd/fulltext.00019605-200201000-00016.htm
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